Impact of Portal Flow on the Prognosis of Children With Congenital Portosystemic Shunt: A Multicentric Observation Study in Japan

Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes. The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in...

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Veröffentlicht in:Journal of pediatric surgery 2024-09, Vol.59 (9), p.1791-1797
Hauptverfasser: Uchida, Hajime, Shinkai, Masato, Okuyama, Hiroomi, Ueno, Takehisa, Inoue, Mikihiro, Yasui, Toshihiro, Hiyama, Eiso, Kurihara, Sho, Sakuma, Yasunaru, Sanada, Yukihiro, Taketomi, Akinobu, Honda, Shohei, Wada, Motoshi, Ando, Ryo, Fujishiro, Jun, Yoshida, Mariko, Yamada, Yohei, Uchida, Hiroo, Tainaka, Takahisa, Kasahara, Mureo
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container_issue 9
container_start_page 1791
container_title Journal of pediatric surgery
container_volume 59
creator Uchida, Hajime
Shinkai, Masato
Okuyama, Hiroomi
Ueno, Takehisa
Inoue, Mikihiro
Yasui, Toshihiro
Hiyama, Eiso
Kurihara, Sho
Sakuma, Yasunaru
Sanada, Yukihiro
Taketomi, Akinobu
Honda, Shohei
Wada, Motoshi
Ando, Ryo
Fujishiro, Jun
Yoshida, Mariko
Yamada, Yohei
Uchida, Hiroo
Tainaka, Takahisa
Kasahara, Mureo
description Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes. The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P 
doi_str_mv 10.1016/j.jpedsurg.2024.05.008
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The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P &lt; 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011). Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic. Level III. •What is currently known about this topic? A congenital portosystemic shunt is a rare disease with diverse manifestations and is divided into intra- and extrahepatic shuts. However, it has been mainly mentioned in case reports or small series with short-term follow-up.•What new information is contained in this article? 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The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P &lt; 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011). Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic. Level III. •What is currently known about this topic? A congenital portosystemic shunt is a rare disease with diverse manifestations and is divided into intra- and extrahepatic shuts. However, it has been mainly mentioned in case reports or small series with short-term follow-up.•What new information is contained in this article? 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The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P &lt; 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011). Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic. Level III. •What is currently known about this topic? A congenital portosystemic shunt is a rare disease with diverse manifestations and is divided into intra- and extrahepatic shuts. However, it has been mainly mentioned in case reports or small series with short-term follow-up.•What new information is contained in this article? 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subjects Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Japan - epidemiology
Liver Transplantation
Male
Portal hypertension
Portal vein
Portal Vein - abnormalities
Portopulmonary hypertension
Portosystemic shunt
Prognosis
Retrospective Studies
Vascular Malformations - epidemiology
Vascular Malformations - surgery
title Impact of Portal Flow on the Prognosis of Children With Congenital Portosystemic Shunt: A Multicentric Observation Study in Japan
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